Recent Advances in Castleman Disease.
Castleman disease
HHV-8
Multicentric CD
Siltuximab
Unicentric CD
Journal
Oncology research and treatment
ISSN: 2296-5262
Titre abrégé: Oncol Res Treat
Pays: Switzerland
ID NLM: 101627692
Informations de publication
Date de publication:
2022
2022
Historique:
received:
06
05
2022
accepted:
15
08
2022
pubmed:
12
10
2022
medline:
3
11
2022
entrez:
11
10
2022
Statut:
ppublish
Résumé
Castleman disease (CD) encompasses a spectrum of rare disorders with characteristic histopathological features. Unicentric CD (UCD) is a benign, local hyperplasia of lymphoid tissue that is usually curable. Multicentric CD (MCD) manifests as a potentially life-threatening systemic disease with complex symptomatology which is mostly due to an overproduction of interleukin-6 (IL-6) or dysregulation of IL-6-related signaling pathways. From a therapeutic perspective, it is important to distinguish idiopathic MCD (iMCD) from those cases that are associated with the human herpesvirus-8 (HHV-8 + MCD). During recent years, it has become increasingly clear that even HHV-8-negative MCD is not a homogeneous entity and that there are clinically distinct variants. International consensus guidelines for diagnosis and treatment have been developed for iMCD and UCD. We herein summarize recent advances in diagnosis, treatment, and novel insights into the pathogenesis of this disease.
Sections du résumé
BACKGROUND
BACKGROUND
Castleman disease (CD) encompasses a spectrum of rare disorders with characteristic histopathological features. Unicentric CD (UCD) is a benign, local hyperplasia of lymphoid tissue that is usually curable. Multicentric CD (MCD) manifests as a potentially life-threatening systemic disease with complex symptomatology which is mostly due to an overproduction of interleukin-6 (IL-6) or dysregulation of IL-6-related signaling pathways. From a therapeutic perspective, it is important to distinguish idiopathic MCD (iMCD) from those cases that are associated with the human herpesvirus-8 (HHV-8 + MCD).
SUMMARY
CONCLUSIONS
During recent years, it has become increasingly clear that even HHV-8-negative MCD is not a homogeneous entity and that there are clinically distinct variants. International consensus guidelines for diagnosis and treatment have been developed for iMCD and UCD.
KEY MESSAGES
CONCLUSIONS
We herein summarize recent advances in diagnosis, treatment, and novel insights into the pathogenesis of this disease.
Identifiants
pubmed: 36219975
pii: 000526640
doi: 10.1159/000526640
doi:
Substances chimiques
Interleukin-6
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
693-704Informations de copyright
© 2022 S. Karger AG, Basel.